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Role of Epithelial Cells in Idiopathic Pulmonary Fibrosis

From Innocent Targets to Serial Killers

Dirección de Investigación, Instituto Nacional de Enfermedades Respiratorias, México City; and Facultad de Ciencias, Universidad Nacional Autónoma de México, México D.F., Mexico

Correspondence and requests for reprints should be addressed to Dr. Moisés Selman, Instituto Nacional de Enfermedades Respiratorias, Tlalpan 4502 CP 14080, México DF, México. E-mail: moiselman{at}salud.gob.mx

ABSTRACT

Idiopathic pulmonary fibrosis (IPF), a progressive and relentless lung scarring of unknown etiology, has been recognized as the most lethal interstitial lung disease. Despite the growing interest in IPF, the precise molecular mechanisms underlying the development of fibrosis and leading to the irreversible destruction of the lung are still unknown. Recently, it has been proposed that IPF, instead of being a chronic inflammatory disorder, results from multiple cycles of epithelial cell injury and activation. In turn, active alveolar epithelial cells provoke the migration, proliferation, and activation of mesenchymal cells with the formation of fibroblastic/myofibroblastic foci and the exaggerated accumulation of extracellular matrix, mirroring abnormal wound repair. In this article, some characteristics of the alveolar epithelium are briefly outlined, and the fibrogenic mechanisms specifically operated by active abnormal epithelial cells are examined.

Key Words: epithelial cells • idiopathic pulmonary fibrosis • lung fibrosis pathogenesis

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